Seizure Freedom in Children With Pathology-Confirmed Focal Cortical Dysplasia.

OBJECTIVE: We evaluated the temporal course of seizure outcome in children with pathology-confirmed focal cortical dysplasia and explored predictors of sustained seizure freedom. METHODS: We performed a single-center retrospective study of children ≤ 18 years who underwent resective surgery from January 1, 2000 through December 31, 2012 and had pathology-proven focal cortical dysplasia. Surgical outcome was classified as seizure freedom (Engel class I) or seizure recurrence (Engel classes II-IV). Fisher exact and nonparametric Wilcoxon ranksum tests were used, as appropriate. Survival analysis was based on seizure-free outcome. Patients were censored at the time of seizure recurrence or seizure freedom at last follow-up. RESULTS: Thirty-eight patients were identified (median age at surgery, 6.5 years; median duration of epilepsy, 3.3 years). Median time to last follow-up was 13.5 months (interquartile range, 7-41 months). Twenty patients (53%) were seizure free and 26 patients (68%) attained seizure freedom for a minimum of 3 months. Median time to seizure recurrence was 38 months (95% confidence interval, 6-109 months), and the cumulative seizure-free rate was 60% at 12 months (95% confidence interval, 43%-77%). Clinical features associated with seizure freedom at last follow-up included older age at seizure onset (P = .02), older age at surgery (P = .04), absent to mild intellectual disability before surgery (P = .05), and seizure freedom for a minimum of 3 months (P < .001). CONCLUSION: Favorable clinical features associated with sustained seizure freedom included older age at seizure onset, older age at surgery, absent or mild intellectual disability at baseline, and seizure freedom for a minimum of 3 months.

Outcome After Therapeutic Hypothermia in Term Neonates With Encephalopathy and a Syndromic Diagnosis.

The large randomized, controlled trials of therapeutic hypothermia for hypoxic-ischemic encephalopathy excluded neonates with congenital disorders. The objective of this study was to report our experience using hypothermia in neonates with signs of hypoxic-ischemic encephalopathy and a syndromic disorder or brain anomaly. Subjects were identified from a database of neonates admitted to the Neuro-Intensive Care Nursery at University of California, San Francisco. Of 169 patients fulfilling criteria for hypothermia, 8 (5%) had a syndromic disorder and were cooled per guidelines for nonsyndromic neonates. Perinatal characteristics of infants with and without syndromic disorder were not significantly different. Overall outcome was poor: 38% had evidence of acute hypoxic-ischemic injury, 3 subjects died, and 2 survivors had low developmental quotient (ie, 25). The risk versus benefit of therapeutic hypothermia for hypoxic-ischemic encephalopathy among neonates with congenital brain malformations or syndromic diagnoses is uncertain.

Comorbidities in infants with obstructive sleep apnea.

STUDY OBJECTIVE: The clinical characteristics of obstructive sleep apnea (OSA) in infants have been insufficiently characterized. Our aim was to describe identifiable comorbidities in infants with obstructive sleep apnea, which may assist in recognizing these patients earlier in their disease course and help improve management. METHODS: This was a single-center, retrospective study involving infants 0-17 months of age with a diagnosis of OSA on the basis of clinical features and nocturnal polysomnography (PSG) at the Mayo Clinic Center for Sleep Medicine between 2000 and 2011. Patients were excluded if they had central apnea accounting for greater than 50% of respiratory events. OSA severity was determined by the apnea-hypopnea index (AHI). RESULTS: One hundred thirty-nine patients were included. Based upon the AHI, they were subdivided into mild (AHI <5; 30%), moderate (AHI 5-9; 30%), or severe (AHI >10; 40%) categories. Comorbidities included gastroesophageal reflux in 95/139 (68%), periodic limb movements in sleep in 59/139 (42%), craniofacial abnormalities in 52/139 (37%), neuromuscular abnormalities in 47/139 (34%), prematurity in 41/139 (29%), genetic syndromes in 41/139 (29%), laryngomalacia / tracheomalacia in 38/139 (27%), and epilepsy in 23/139 (17%) of subjects. Severity of OSA correlated with prematurity, having a genetic syndrome, or neuromuscular abnormality. Multispecialty evaluation was needed for 119/139 (86%). CONCLUSION: Comorbidities in infants with OSA differ from those of older children. Based upon the comorbidities identified in our study population, it appears that appropriate management of infants with OSA requires a multidisciplinary approach involving genetics, gastroenterology, pulmonology, otolaryngology, neurology, and general pediatrics.

Chemical meningitis: a rare presentation of Rathke's cleft cyst.

Rathke's cleft cysts (RCC) are usually benign, sellar and/or suprasellar lesions originating from the remnants of Rathke's pouch. Rarely, RCC can present with chemical meningitis, sellar abscess, lymphocytic hypophysitis, or intracystic hemorrhage. We describe an unusual presentation of RCC in which the patient presented with a clinical picture of chemical meningitis consisting of meningeal irritation, inflammatory cerebrospinal fluid profile, and enhancing pituitary and hypothalamic lesions, in addition to involvement of the optic tracts and optic nerve.

Clostridium septicum brain abscesses in a premature neonate.

Brain abscesses in neonates are typically caused by Gram-negative organisms. There are no previously described cases caused by Clostridium septicum. We present a case of a premature male infant who developed recurrent episodes of suspected necrotizing enterocolitis followed by brain abscesses, cerebritis and ventriculitis caused by C. septicum.